Aortopulmonary Fistula: A Rare Complication of an Aortic Aneurysm

In a patient with degenerative disease of the thoracic aorta, an aortopulmonary fistula with an aortic aneurysm after trauma is a rare occurrence. Few cases of successful surgical management have been reported. Aortopulmonary fistula should be suspected in a patient who has an aortic aneurysm and exhibits signs of congestive heart failure. Herein, we report the case of a 50-year-old man who underwent surgical repair of an ascending aortic aneurysm with fistula into the main pulmonary artery. Early diagnosis and prompt surgical intervention were crucial to the successful outcome.Key words: Aortic aneurysm, thoracic/complications/diagnosis; arterio-arterial fistula/etiology/surgery; fistula/surgery; pulmonary artery/abnormalities/pathology/surgery; vascular surgical proceduresThe natural course of thoracic aortic aneurysms usually involves acute aortic dissection or free rupture of the aneurysm rather than congestive heart failure. An aortic aneurysm with aortopulmonary fistula after trauma is unusual. Few cases of successful surgical management have been reported. Aortopulmonary fistula should be suspected in a patient who has an aortic aneurysm and shows signs of congestive cardiac failure. Early diagnosis and prompt surgical intervention are crucial to a successful outcome. Here, we report the case of a 50-year-old man with this combination of conditions.     

Natural history of a dilated ascending aorta after aortic valve replacement.

BACKGROUND: Little information is available regarding the incidence of aortic dissection or rupture in patients with a dilated ascending aorta after aortic valve replacement (AVR). The present clinical study aimed to demonstrate the incidence of aortic complications after AVR in patients with a dilated ascending aorta and to clarify those risk factors associated with the progression of a dilated ascending aorta or late aortic events. METHODS AND RESULTS: A total of 35 patients with a dilated ascending aorta at the time of AVR were enrolled. A dilated ascending aorta was defined as 40 mm or greater in diameter by preoperative computed tomography or operative findings. The baseline ascending aorta diameter ranged from 40 to 55 mm with a mean of 44.8+/-4.4 mm. There was a high frequency of bicuspid valve disease in patients with a dilated ascending aorta (57%). The mean follow-up interval was 8.1+/-3.5 years (range: 2.3-13). Aortic events occurred in 5 patients (aortic dissection in 1, rupture in 2, reoperation in 2) during the follow-up. One aortic dissection developed at a baseline aortic size of 42 mm, whereas 2 aortic ruptures occurred at baseline aortic sizes of 47 mm and 50 mm. There was no statistically significant univariate association between any of the patient clinical characteristics and late aortic events or ascending aortic progression. CONCLUSION: Although the clinical course of patients with a dilated ascending aorta is unpredictable, aortic events may occur even in patients with a baseline aortic diameter of <50 mm. Therefore, preventive aortic surgery at the time of AVR should be considered to prevent aortic dissection or rupture in patients with an even slightly dilated ascending aorta with a diameter of 40 to 50 mm, unless the patient has a high operative risk or older age.     

Aortic dissection presenting as pericarditis

In five patients with aortic dissection, signs and/or symptoms of pericarditis were part of the early manifestations of the aortic disease. Signs of inflammatory pericarditis were noted clinically in four patients and were found at autopsy in one. In the three nonoperated patients who died of aortic rupture leading to fatal hemopericardium, symptoms of pericarditis preceded fatal rupture of the aorta by four to five days. A fourth patient died after surgical repair of aortic dissection 35 days after the onset of pericarditis. In the fifth patient, manifestations of chronic constrictive pericardial disease occurred over a period of seven months after which old aortic dissection was first identified. In each case, the internal tear of classic aortic dissection was located in the ascending aorta. Microscopic evidence of cystic medial necrosis of the aorta was present in each case. In each of two cases, there was a congenital bicuspid aortic valve. The phenomenon observed represents acute aortic dissection in which slow penetration of blood into the pericardial space caused inflammatory pericarditis. The interval between the onset of pericarditis and rupture of the aorta may allow sufficient time for appropriate diagnosis and potentially lifesaving treatment of the aortic disease.     

Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy

The ascending aorta or pulmonary trunk in congenital heart disease may dilate out of proportion to hemodynamic or morphogenetic expectations, may become aneurysmal, and may rupture. A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Congenital heart diseases such as single ventricle, truncus arteriosus, transposition of the great arteries and tetralogy of Fallot are also associated with aortic medial abnormalities. Aortic regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation which may lead to regurgitation and predispose to dissection and rupture which can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities such as pulmonary atresia, right aortic arch and a history of an aorto-pulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker administration for limiting progressive dilatation of the aortic root in patients with congenital heart disease and repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and or prevent the risk of aortic dissection. Meticulous follow-up of the aortic root after repair, tetralogy of Fallot are thus recommended.     

Diagnosis of aortic pseudoaneurysm by echocardiography.

The diagnosis of pseudoaneurysm of the ascending aorta is of paramount importance because of its propensity to rupture. As the frequency of surgical procedures involving the aortic root and valve increases, an increase in the incidence of aortic pseudoaneurysm may be anticipated. We recently studied a patient who developed pseudoaneurysm of the ascending aorta following repair of a Type I aortic dissection, utilizing a composite graft. Two-dimensional echocardiography with color flow and pulsed Doppler imaging showed a large perigraft cavity communicating with the aorta. Echocardiography provides a safe noninvasive diagnostic tool for the evaluation of the aorta postoperatively and for screening for pseudoaneurysm formation in the follow-up period.     

Repair of a false aneurysm of the aortic arch

A 66-year-old man having previously undergone repair of aneurysms of the ascending, transverse and infrarenal aorta, presented with a large false aneurysm of the aortic arch. Successful repair of the aneurysm was achieved under a state of profound hypothermia and circulatory arrest. The patient remains well and free from aortic aneurysmal disease two years after surgery.     

Anomalous right coronary artery angioplasty complicated by intramural hematoma in the ascending aorta

An iatrogenic intramural hematoma (IMH) localized in the ascending aorta is a rare and potentially life-threatening complication following percutaneous coronary intervention (PCI). We describe the case of an ascending aortic IMH after the PCI of an anomalous right coronary artery. Early extension of the hematoma was observed during transesophageal echocardiography; the patient underwent successful surgical repair.     

Aorto-right artrial fistula: a rare complication of aortic dissection

We describe the successful surgical repair of an acute aortic dissection that had caused an aorto-right atrial fistula in a 67-year-old man. The patient was admitted to the hospital on an emergency basis because of severe heart failure. The diagnosis of acute aortic dissection with rupture into the right atrium was confirmed by use of intraoperative transesophageal echocardiography, although rupture of a sinus of Valsalva aneurysm into the right atrium had been suggested initially by 2-dimensional and Doppler transthoracic echocardiography. At surgery, we found the patient to have aortic arch dissection with complete separation of the right coronary artery from the sinus of Valsalva and a false lumen that had ruptured into the right atrium. The aortic arch was repaired directly. The ascending aorta was successfully replaced with a composite graft. Aortic dissection with rupture into the right atrium is extremely rare and leads to death rapidly. As shown in this case, such a condition might be mistaken for an aneurysmal rupture of the sinus of Valsalva, with use of transthoracic echocardiography alone. Transesophageal echocardiography is a useful noninvasive method to further define or confirm the diagnosis. Early surgical intervention is necessary in patients with this condition to prevent profound shock and end-organ failure.     

A rare case of Marfan syndrome with involvement of the thoracoabdominal aorta--its surgical treatment]

A 49-year-old woman with classic manifestations of Marfan's syndrome, having previously been submitted to two surgical procedures for ascending aorta and aortic valve replacement, was admitted with diagnosis of a large dissecting thoracoabdominal aortic aneurysm, with indication for surgical repair. The patient underwent a successful reconstruction of the aorta by means of the "simplified technique" introduced by ourselves in 1984, as an alternative to the conventional S. Crawford's "inclusion technique", for the management of thoracoabdominal aortic aneurysms. Assessed two years after the operation, the patient was found to be in excellent clinical condition and a control angiography showed the vascular prosthesis and its side branches working properly. This unusual aortic complication of Marfan's syndrome and the usefulness of the unique and original technique employed to replace the thoracoabdominal aorta justify the presentation of this clinical case.     

Interrupted aortic arch in an adult single-stage extra-anatomic repair

Interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment. To our knowledge, the world medical literature contains only 12 reports of isolated interrupted aortic arch diagnosed in adults. Nine of these patients underwent successful surgical repair, but 1 died during the early postoperative period. We describe a 10th successful surgical repair, which involved a 42-year-old woman who had an asymptomatic type B interrupted aortic arch (characterized by interruption between the left subclavian and left carotid arteries). We performed a single-stage extra-anatomic repair by placing a 16-mm extra-anatomic Dacron graft between the ascending and descending portions of the thoracic aorta and by interposing a 7-mm extra-anatomic Dacron graft between the 16-mm graft and the left subclavian artery. The patient recovered uneventfully and continued to do well 6 months later.     

Aortic valve regurgitation caused by blunt chest injury.

Aortic valve regurgitation is an uncommon consequence of closed chest injury. It is caused by damage to the valve apparatus (ruptured cusp) or when subadventitial rupture of the ascending aorta causes prolapse of a subjacent valve cusp. Aortic valve regurgitation was detected in 4 patients (2 men and 2 women, 30 to 65 years old) who had sustained multiple injuries in road accidents 1 week to 30 years before. Three had subadventitial rupture of the ascending aorta and one had isolated rupture of the noncoronary cusp of the aortic valve. The mechanism responsible for the damage was believed to be a consequence of multiple chest lesions (right costal flap, sternal fracture, pulmonary contusion). It is difficult to diagnose and treat aortic regurgitation in patients with multiple injuries. Three patients had repair operations and the remaining patient needed valve replacement. If aortic regurgitation is haemodynamically well tolerated, the operation should be postponed until the patients have recovered from their other injuries. The results in these 4 patients and in other reported cases indicate that operations can be performed soon after the acute phase.     

Endovascular repair of ascending aortic pseudoaneurysm: technical considerations of a common carotid artery approach using the Zenith aortic cuff endograft.

PURPOSE: To present a technique for endovascular treatment using Zenith aortic cuff extenders delivered via a left common carotid artery (CCA) approach in a patient with a large symptomatic ascending aortic pseudoaneurysm. CASE REPORT: A 78-year-old man with recent stroke developed worsening exertional dyspnea and chest pain 4 years following coronary artery bypass grafting. Imaging demonstrated a bovine arch and an 8-x12-cm ascending aortic pseudoaneurysm that was compressing the pulmonary arteries. The treatment strategy was to deliver a Zenith aortic cuff to seal the ascending aortic pseudoaneurysm via a left CCA approach. With the patient under general anesthesia, the left CCA was exposed and a transverse arteriotomy was made to introduce the Zenith aortic cuff sheath; the distal CCA was clamped to prevent catheter-related embolization. With its nosecone removed, a 32-x36-mm Zenith aortic cuff was delivered to the ascending aorta via the left CCA and positioned under transient cardiac arrest initiated with intravenous adenosine. A total of 3 Zenith aortic cuffs were placed in the ascending aorta to successfully exclude the pseudoaneurysm. The patient tolerated the procedure well; follow-up imaging showed successful pseudoaneurysm exclusion without endoleak. CONCLUSION: Ascending aortic pseudoaneurysm is a formidable clinical challenge due in part to the significant operative stress in a conventional surgical repair, as well as limited endovascular treatment options. Because there are no approved endovascular devices for ascending aortic aneurysm repair, clinicians may have to rely on endograft components designed for abdominal aortic aneurysms to treat lesions in the ascending aorta.     

Aortic valve repair of congenital bicuspid aortic valve associated with aneurysm of the ascending aorta

Two unusual cases of congenital bicuspid aortic valve associated with aneurysm of the ascending aorta are reported. One patient with a 7-cm ascending aortic dilatation and aortic regurgitation (AR) (II/IV), and another with a 6-cm ascending aorta and AR (III/IV), presented for treatment. Replacement of the ascending aorta and aortic valve repair were performed in both cases. Aortic valve repair included resection of the raphe, leaflet plication and subcommissural annuloplasty. Both patients had satisfactory results in the early postoperative period. Despite the promising outcomes after surgery in these patients, long-term changes in valve function and durability remain unknown. Additional close observation and monitoring are required before the procedure can be recommended as the standard of care.     

Spontaneous rupture of the ascending aorta: case report and review.

Spontaneous nontraumatic rupture of the ascending aorta occurred in a hypertensive patient. The clinical findings suggested acute aortic dissection, and echocardiography showed a large pericardial effusion. Computed tomography scanning did not indicate aortic dissection, but aortography in 3 projections revealed an area of intimal disruption similar to the niche of an ulcer. The patient underwent replacement of the ascending aorta and proximal aortic arch, and the postoperative course was uneventful.     

Spontaneous rupture of the ascending aorta. A surgically treated case

Rupture of the ascending aorta may follow thoracic trauma or complicate an aortic aneurysm or acute dissection. It is otherwise extremely rare. The authors report a case of spontaneous rupture of the ascending aorta occurring in a patient with a pre-existing incomplete rupture of the ascending aorta, and treated surgically. The clinical presentation was of acute dissection with pericardial effusion. This diagnosis was excluded by aortography with multiple views which showed abnormalities of the aortic wall: an abnormal notch, continuity of the internal wall and extravasation of the contrast medium. These abnormalities are often minimal but should be recognised and surgery proposed as this is the only chance of a favourable outcome.     

Contained ascending aortic rupture disguised as a right atrial mass

Transesophageal echocardiography can incidentally detect cardiac masses. A right atrial mass has many possible causes, including metastatic extension from various solid organs, malignant melanoma, mediastinal cysts, hematoma, myxoma, lipoma, and artifact.Herein, we report our discovery of a right atrial mass during the initial intraoperative transesophageal echocardiographic examination of a patient who was undergoing repair of an ascending aortic aneurysm. The mass, with a smooth border and homogenous grayscale density, showed no flow during color-flow Doppler echocardiography. Further examination revealed tricuspid annular dilation and severe tricuspid insufficiency. When the mediastinum was opened, we found a large extracardiac hematoma along the right atrium within the pericardial space. These findings suggested an acute, contained rupture of the ascending aorta. After the hematoma was evacuated, imaging revealed complete resolution of the mass and reduction of tricuspid insufficiency. The patient underwent the scheduled aortic repair and was discharged from the hospital in good condition.In order to ensure an accurate diagnosis and to gain appropriate clinical guidance that can lead to a successful outcome, we suggest performing a thorough echocardiographic investigation to identify any such mass before planned surgery proceeds.     

Hybrid endovascular and off-pump open surgical treatment for synchronous aneurysms of the aortic arch, brachiocephalictrunk, and abdominal aorta

A 71-year-old patient was admitted for synchronous aneurysms of the aortic arch, brachiocephalic trunk, and juxtarenal abdominal aorta involving the iliac arteries. The patient first underwent open surgical repair of the juxtarenal abdominal aortic aneurysm by means of aorto-bifemoral bypass. Three months later, he underwent off-pump surgical repair of the aneurysm of the brachiocephalic trunk and bypass grafting from the ascending aorta to the brachiocephalic trunk and the left common carotid artery, followed by successful exclusion of the aneurysm of the aortic arch by deployment of a Zenith TX1 custom-made endograft, inserted through a limb of the aorto-bifemoral graft. Combined endovascular and open surgical treatment is an appealing new alternative to open surgical repair for complex aortic diseases. Debranching of the aortic arch enables endovascular grafting in this area, thereby avoiding cardiopulmonary bypass and circulatory arrest. Staged and simultaneous procedures should be considered for the treatment of complex aortic diseases even in poor-risk patients; however due to the investigative characteristics of these procedures, patient selection and postoperative follow-up should be carried out with utmost attention.     

Type A quadricuspid aortic valve and ascending aorta aneurysm: a rare combination

Quadricuspid aortic valve (QAV) is a rare congenital anomaly associated with aortic valve insufficiency and significant morbidity, and requires the replacement or, rarely, the repair of the malfunctioning heart valve. A QAV associated with an ascending aorta aneurysm is an extremely rare anatomic combination with a hypothetical, but not clear, shared embryological etiology. To date, only two cases of type B QAV with ascending aorta aneurysm have been reported. Herein is described the first ever case of a 38-year-old male suffering from severe symptomatic aortic valve regurgitation due to a type A QAV, associated with an ascending aorta aneurism, who underwent a successful combined replacement of the aortic valve and ascending aorta.     

Beneficial effect of BioGlue surgical adhesive in repair of iatrogenic aortic dissection

We report the benefits of using BioGlue surgical adhesive to repair an iatrogenic aortic rupture and dissection that resulted from cannulation of the ascending aorta during open-heart surgery.     

Chirurgische Behandlung kardiovaskulärer Manifestationen des Marfan-Syndroms

Patients with Marfan's syndrome suffer mainly from the cardiovascular manifestations of the disease, in particular the acute dissection or rupture of the dilated aorta. Due to improved diagnostic and early surgical intervention the life expectancy of these patients could be considerably improved. However, rupture is still the most frequent and dissection the second frequent cause of death. Life threatening complications of aortic dissection are pericardial effusion, aortic insufficiency and malperfusion syndrome, due to obstruction of aortic branches. Dissection of the ascending aorta is treated by implantation of a valved conduit with reimplantation of the coronary arteries. Some surgeons favor the complete replacement of the ascending aorta with preservation of the aortic valve, although long-term results show some development of aortic insufficiency after this procedure. Based on the experience of the last years, most surgeons prefer the prophylactic replacement of the aorta in Marfan patients, i.e. before complications have occurred. A special treatment algorithm helps to define the indication for the operative treatment in different manifestations of the disease. The low mortality of the elective replacement of the ascending aorta in contrast to replacement in emergency cases speaks in favor of the early operative treatment. For the long-term prognosis of the patient a closed and continuous cardiologic surveillance is mandatory. The patient should be close to a center with the necessary diagnostic tools and with sufficient experience with the medical and surgical treatment, in order to further improve the life expectancy in the future.